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Histiocytosis X in Adult With Predominant Pulmonary Manifestation

Arch Intern Med. 1964;113(2):261-266. doi:10.1001/archinte.1964.00280080097018.
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Introduction  Histiocytosis X, a proliferation of histiocytes of indefinite etiology, is classified into localized (eosinophilic granuloma) and diffuse, acute (Letterer-Siwe) or chronic (Hand-Schüller-Christian) varieties. The cause of such a proliferation remains unknown.1 Inflammatory, allergic, or neoplastic origins are suggested, but as yet remain unproved.2,3These three forms, although clinically different, overlap each other, and various combinations or "transitional" forms producing bizarre manifestations have been described.This report deals with a fulminant disseminated histiocytosis with predominant pulmonary pathology, which appeared in a young woman. Such a combination is exceptional. To our knowledge only one similar case has been reported in the literature.4

Report of a Case  A 22-year-old housewife, previously healthy, was hospitalized because of high fever, headache, weakness, and productive cough of ten days' duration. On physical examination, a severely ill, thin woman was found. The breathing was rapid and shallow and mild cyanosis was seen. Blood


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